Phenylketonuria (PKU) is an autosomal recessive genetic disorder characterized by a deficiency in the enzyme phenylalanine hydroxylase (PAH). This enzyme is necessary to metabolize the amino acid phenylalanine to the amino acid tyrosine. When PAH is deficient, phenylalanine accumulates and is converted into phenylpyruvate (also known as phenylketone), which is detected in the urine.
The term "phenylketonuric" is an individual whose body cannot process certain substances (phenylalanine) into the amino acid tyrosine. This can cause a toxic buildup of phenylketone (aka phenylpyruvate) in the body tissues.The condition is a genetic defect that is ordinarily treated by dietary changes. It has gained prominence due to warnings for the artificial sweetener aspartame (Nutrasweet, Equal). Because the human body breaks down the sweetener into other substances, including phenylalanine, the consumption of the product could elevate the level of that chemical in phenylketonuric individuals.
Essential amino acids are those the body cannot produce. There are 20 basic amino acids. There are eight essential amino acids unless the individual is a phenylketonuric. There are nine essential amino acids for phenylketonurics as they cannot convert phenylalanine to tyrosine. Essential amino acids have to be taken into the body by eating. This answer is for informational purposes only. All individuals should ask advice of their physician.
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If you have been tested and found to NOT be phenylketonuric, go for it. If you ARE phenylketonuric, any amount of aspartame can cause serious problems. Laws in most states require that infants be tested for phenylketonurea before they are allowed out of the hospital. If you were born in a hospital, you have been tested. If you have eaten a banana and not gone into siezures or suffered serious brain damage, you are NOT phenylketonuric and you could safely consue the amount of aspartame that is in 12 diet cokes every day. On the other hand, you SHOULD be concerned with the phosphoric acid and some of the other chemicals that are in soft drinks, and restrict your intake of them. === === Note: This information required a Freedom Of Information Act request to pry it from the reluctant hands of the FDA. Nutrasweet (brand name for Aspartame) was not approved until 1981, in dry foods. For over eight years the FDA refused to approve it because of the seizures and brain tumors this drug produced in lab animals. The FDA continued to refuse to approve it until President Reagan took office (a friend of Searle) and fired the FDA Commissioner who wouldn't approve it. Dr. Arthur Hull Hayes was appointed as commissioner. Even then there was so much opposition to approval that a Board of Inquiry was set up. The Board said: "Do not approve aspartame". Dr. Hayes OVERRULED his own Board of Inquiry. Shortly after Commissioner Arthur Hull Hayes, Jr., approved the use of aspartame in carbonated beverages, he left for a position with G.D. Searle's Public Relations firm. Long-Term Damage. It appears to cause slow, silent damage in those unfortunate enough to not have immediate reactions and a reason to avoid it. It may take one year, five years, 10 years, or 40 years, but it seems to cause some reversible and some irreversible changes in health over long-term use. METHANOL (AKA WOOD ALCOHOL/POISON) (10% OF ASPARTAME) Methanol/wood alcohol is a deadly poison. People may recall that methanol was the poison that has caused some "skid row" alcoholics to end up blind or dead. Methanol is gradually released in the small intestine when the methyl group of aspartame encounter the enzyme chymotrypsin. The absorption of methanol into the body is sped up considerably when free methanol is ingested. Free methanol is created from aspartame when it is heated to above 86 Fahrenheit (30 Centigrade). This would occur when aspartame-containing product is improperly stored or when it is heated (e.g., as part of a "food" product such as Jello). Methanol breaks down into formic acid and formaldehyde in the body. Formaldehyde is a deadly neurotoxin. An EPA assessment of methanol states that methanol "is considered a cumulative poison due to the low rate of excretion once it is absorbed. In the body, methanol is oxidized to formaldehyde and formic acid; both of these metabolites are toxic." The recommend a limit of consumption of 7.8 mg/day. A one-liter (approx. 1 quart) aspartame-sweetened beverage contains about 56 mg of methanol. Heavy users of aspartame-containing products consume as much as 250 mg of methanol daily or 32 times the EPA limit. See the nearby links for an official FDA view and an independent assessment.
Essential amino acids are those that the body cannot produce and must be obtained from the diet. On the other hand, non-essential amino acids can be synthesized by the body. Both types of amino acids are crucial for synthesizing proteins and supporting various biological functions in the body.
In humans, disorders of phenylalanine or tyrosine metabolism include phenylketonuria (PKU) and alkaptonuria. PKU is caused by a deficiency in the enzyme phenylalanine hydroxylase, leading to a buildup of phenylalanine in the body. Alkaptonuria is due to a deficiency in homogentisate 1,2-dioxygenase, resulting in the accumulation of homogentisic acid. Both disorders can have serious health consequences if not managed properly.
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